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Michael P. D'Alessandro, M.D.
Peer Review Status: Internally Peer Reviewed
The patient is a 14 year old African-American male who presented at the age of 3 months with dyspnea, cough, tachypnea and failure to thrive. He had an open lung biopsy at 8 months. Since then, he has had repeated respiratory exacerbations. He was on steroids and prophylactic antibiotics and receives oxygen at night.
Clinical Physical Exam:
The patient was thin and almost emaciated. He became short of breath when speaking. Auscultation revealed respiratory crackles. He has 3+ clubbing.
Clinical Differential Diagnosis:
Chest films demonstrated prominent interstitial infiltrates. A chest CT exam showed prominent interstitial fibrosis.
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Images 3, 4 and 5
Imaging Differential Diagnosis:
Idiopathic pulmonary fibrosis.
An open lung biopsy was performed at the age of 8 months.
Pathological examination of the lung biopsy specimen at 8 months revealed proliferative interstitial pneumonia with focal fibromuscular hyperplasia.
Idiopathic Pulmonary Fibrosis
Follow-up and Prognosis:
The patient was awaiting lung transplantation.
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